Evaluation and treatment of Sjögren’s syndrome in focus

Sjögren's syndrome is little known, despite the fact that it is one of the most common chronic rheumatic inflammatory diseases, approximately as common as systemic lupus erythematosus.¹ It is estimated that almost 400,000 Brazilian people suffers from the disease, most of those did not yet get the diagnosis.² In the last decade the number of articles about Sjögren's syndrome was 5 times and 10 times less than systemic lupus erythematosus and rheumatoid arthritis, respectively. Also, in the last decade, biological therapeutic has revolutionized the treatment of rheumatic disease. Now the time has come for finding and testing new targets for treatment of Sjögren's syndrome.

There is a global effort to organize large cohorts and registries, to validate new classification criteria and tools to evaluate disease activity and symptoms, to develop new targets and to test new treatments for Sjögren's syndrome. Recently, new findings in the pathogenesis, diagnosis and treatment were discussed among 260 participants of the 13th International Symposium on Sjögren's syndrome (ISSS), in Bergen, Norway.¹ There, the proposed new ACR-EULAR classification criteria were presented. In spite of not yet being included ultrasound of salivary glands, the new consensus considers all systemic manifestation included in the EULAR-Sjögren's syndrome disease activity index (ESSDAI) when screening Sjögren's syndrome. The definition of disease activity states and clinically meaningful improvement in primary Sjögren's syndrome with ESSDAI and patient-reported indexes (ESSPRI) were essential to design future studies of treatment.^3,4 Biologic agents targeting B cells (such as rituximab and belimumab) and T cell stimulation inhibition have shown promising results. Modulation or inhibition of other targets such as IFN, IL-6 and Toll-like receptor are also currently being investigated. More than new targets, in the future, personalized and cell-based treatment can bring better results.

The current evidence for treating and the urgent need to standardize the treatment for this disease motivate medical and patient organizations to create guidelines for treating Sjögren's syndrome. At the 13th ISSS, Recommendations from Brazilian Society of Rheumatology and from Sjögren's Syndrome Foundation (SSF) were presented. Since last year the EULAR-Task Force is preparing a full guideline.¹

The current issue of the Brazilian Journal of Rheumatology brings two articles about Sjögren's syndrome. One is the evaluation of psychometric properties of ESSPRI in a Brazilian population. Currently there are four validated tools useful for studying Sjógren's syndrome in Brazil (ESSDAI, ESSPRI, PROFAD and FACIT).^5,6

The second article is the Recommendations of Brazilian Society for treatment of Sjögren's syndrome based on a systematic review, including 127 articles. The treatment of glandular involvement was based on a high level of evidence, but most recommendations for systemic manifestations were based on case reports. Especially when good evidence is not available, the recommendations elaborated by a multidisciplinary panel of specialists is an important reference for treating severe manifestations.

Referências

• ¹
  Meeting Abstracts from The 13th International Symposium on Sjögren’s Syndrome Bergen, Norway, May 19-22, 2015. Scandn J of Immunol. 2015;81:328-450.
• ²
  Valim V, Zandonade E, Pereira AM, de Brito Filho OH, Serrano EV, Musso C, et al. Primary Sjögren’s syndrome prevalence in a major metropolitan area in Brazil. Rev Bras Reumatol. 2013;53:24-34.
• ³
  Seror R, Theander E, Brun JG, Ramos-Casals M, Valim V, Dörner T, et al., EULAR Sjögren’s Task Force. Validation of EULAR primary Sjögren’s syndrome disease activity (ESSDAI) and patient indexes (ESSPRI). Ann Rheum Dis. 2015;74:859-66.
• ⁴
  Seror R, Bootsma H, Saraux A, Bowman SJ, Theander E, Brun JG, on behalf of the EULAR Sjögren’s Task Force. Defining disease activity states and clinically meaningful improvement in primary Sjögren’s syndrome with EULAR primary Sjögren’s syndrome disease activity (ESSDAI) and patient-reported indexes (ESSPRI). Ann Rheum Dis. 2014 Dec 5, pii: annrheumdis-2014-206008.
• ⁵
  Serrano EV, Valim V, Miyamoto ST, Giovelli RA, Paganotti MA, Cadê NV. Transcultural adaptation of the “EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI)” into Brazilian Portuguese. Rev Bras Reumatol. 2013;53:483-93.
• ⁶
  Miyamoto ST, Paganotti MA, Serrano ÉV, Giovelli RA, Valim V. Assessment of fatigue and dryness in primary Sjögren’s syndrome: Brazilian version of “Profile of Fatigue and Discomfort - Sicca Symptoms Inventory (short form) (PROFAD-SSI-SF)”. Rev Bras Reumatol. 2015;55:113-22.

Publication Dates

• Publication in this collection
  Sep-Oct 2015